Rare brain disease that causes progressive physical and mental deterioration, leading to death usually within a year of onset. It is caused by the accumulation of infectious abnormal proteins, known as prions, in the brain, causing irreversible damage to nerve cells. There is no known cure for the disease, which accounts for 1–2 deaths per million of population worldwide each year.
CJD is one of a group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs) since they are characterized by the appearance of spongy changes in brain tissue. The mechanism of all TSEs, including scrapie in sheep and bovine spongiform encephalopathy (BSE) in cattle, is thought to be infection by prions, which are known to be able to cross species, as in the development of CJD in humans from BSE in cattle.
Symptoms begin with mild conditions, such as slurred speech and loss of coordination, and become more serious as the disease progresses, eventually causing loss of memory, intellect, and personality before resulting in death. The only way to confirm a diagnosis of CJD is by a biopsy of brain tissue or by post mortem examination of the brain.
Prions are very resilient and are not affected by antibiotics or antiviral medicines. They cannot be removed by cooking: only levels of heat or radiation sufficient to break down proteins can destroy them.
The commonest form of CJD is sporadic CJD, which is thought to result from normal brain proteins spontaneously changing into prions, a process that occurs in some individuals with age. Symptoms usually manifest in the 60–65 age range. There were 83 deaths from sporadic CJD in the UK in 2012.
Variant CJD, or vCJD, was first described in the UK in 1996 and is linked to exposure to and consumption of BSE-infected products. Strict controls on prion-contaminated meat entering the food chain have been very successful in preventing the spread of vCJD. In 2012 there were no recorded deaths from this form of the disease.
Familial or inherited CJD is a very rare condition that affects only one person in 9 million. Affected persons carry a defective gene which causes the formation of prions during adulthood. Symptoms usually develop around the age of 50. There were ten deaths attributed to familial CJD in the UK in 2012.
Latrojenic CJD is the name given to CJD resulting from infection during a medical procedure, such as growth treatment using human growth hormone harvested from the pituitary glands of infected human donors or the use of contaminated surgical instruments. Synthetic alternatives and better medical sterilization procedures have significantly reduced this form of the disease: in 2012 there were five deaths from it in the UK.
BSE: The Debate Goes On
A rare and fatal neurological disorder of humans caused by a ➤prion and characterized by porous lesions in the brain. Symptoms include progressive d
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